Long-term treatment of pulmonary arterial HTN (PAH) in adult patients w/ WHO functional class II-III, including idiopathic & heritable PAH, PAH associated w/ connective tissue disorders, & PAH associated w/ corrected simple congenital heart disease, either as combination therapy in patients insufficiently controlled w/ endothelin receptor antagonist &/or phosphodiesterase type 5 inhibitor, or as monotherapy in patients who are not candidates for these therapies.
Initially 200 mcg bd, approx 12 hr apart (in the morning & in the evening). May be increased in increments of 200 mcg bd, usually at wkly intervals. Max: 1,600 mcg bd.
View Uptravi overdosage for action to be taken in the event of an overdose.
Administration
May be taken with or without food: Take consistently w/ or w/o food. Swallow whole, do not chew/split/crush.
Contraindications
Hypersensitivity. Severe CHD or unstable angina, MI w/in last 6 mth; decompensated cardiac failure if not under close medical supervision, severe arrhythmias; cerebrovascular events (eg, transient ischaemic attack, stroke) w/in last 3 mth; congenital or acquired valvular defects w/ clinically relevant myocardial function disorders not related to pulmonary HTN. Concomitant use of strong CYP2C8 inhibitors eg, gemfibrozil. Severe hepatic impairment (Child-Pugh class C).
Hypotension, monitor BP. Increased heart rate. Hyperthyroidism, perform thyroid function tests as clinically indicated. Pulmonary veno-occlusive disease, discontinue if confirmed. Hepatic impairment, once-daily regimen recommended in patients w/ Child-Pugh class B, avoid use in severe hepatic impairment. Renal impairment, severe renal impairment, caution should be exercised during dose titration. May affect ability to drive & use machines. Avoid use during pregnancy. Not recommended during lactation. Paed <18 yr. Elderly >75 yr.
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